. Author manuscript; available in PMC: 2023 Dec 30.

Published in final edited form as: Clin Geriatr Med. 2019 May 9;35(3):349–367. doi: 10.1016/j.cger.2019.03.006

Table 1.

Commonly recognized sickle-related complications

Complication	Definition	Comments
Pain	Acute pain, most commonly in the long bones, chest, back Chronic pain is pain lasting for>3mo	Acute episodic pain occurs throughout life. Adults with SCD experience pain on>54% of days¹⁰⁰ Chronic pain occurs in>50% and about 40% of adults with SCD take daily opioids¹⁰¹
Anemia	Acute anemia: A decline in hemoglobin of 2g/dL or more from steady-state values Chronic anemia: severity increases with age and major contributor to insidious organ dysfunction	Variety of causes, including infection (transient red cell aplasia most commonly caused by acute parvo virus B19): acute hemolysis accompanying severe VOC. delayed transfusion reaction Other common causcs of anemia to consider in older patients: iron deficiency. B12 deficiency, hypothyroidism, and anemia of inflammation
ACS	Acute onset of respiratory symptoms with features similar to pneumonia	Although less frequent, outcome is more severe in adults³⁰
PH	Mean pulmonary artery pressure of>25mm Hg at rest, measured by right heart catheterization	6.0%–10.4% prevalence during adulthood^{35, 36, 37, 102, 103}
Heart failure	Most commonly left ventricular failure	Universal to some degree in adults older than age 30³⁹
Chronic sickle lung disease	Progressive restrictive lung function deficit with fibrotic changes on high-rcsolution computed tomographic (CT) scan	Restrictive lung defects seen in>70% adults¹⁰⁴
VTE	DVT detected by duplex ultrasonography, ventilation perfusion scintigraphy, or CT pulmonary angiography	11.2%−13.1% cumulative incidence rate in adulthood. High recurrence rates ~30%–40%. Contribution toward chronic thromboembolic PH. Consider indefinite anticoagulation.^{40, 41} Routine VTE prophylaxis during hospitalization and periods of increased thrombosis risk
Stroke	Acute cerebrovascular accident	Acute ischemic and hemorrhagic stroke. Hemorrhagic stroke affects both children and adults. 3-fold more in adults.¹⁰⁵ Exclude traditional risk factors for ischemic stroke: hypertension, diabetes mellitus. hypcrlipidemia. atrial fibrillation and renal disease¹⁰⁶
SCI	Clinically silent lesions of 3 mm or more on MRI scanning	Important contributing factor to neurocognitive deficits: 53% in adults⁵⁸
Acute renal injury	Acute deterioration in renal function	Triggers include pain crisis, during ACS. acute drop in hemoglobin as in transient red cell aplasia. Often occurs as part of multiorgan failure
Renal failure	Deteriorating renal function, reduced concentrating ability, proteinuria, and progressive renal failure	Advanced disease (stage III-1V) in 4%−18% of adults⁴⁸
Priapism	Unwanted painful and sustained erection of the penis for more than 4h. often recurrent or persistent	20%−89% lifetime prevalence in boys and men¹⁰⁷
AVN of bones	AVN of any bone, most commonly the femoral head and shoulder joint	>20% lifetime prevalence of symptomatic disease. Increased prevalence of asymptomatic disease¹⁰⁸
Leg ulceration	Most commonly around the malleolar regions	>14% lifetime prevalence ^{109, 110}
Cholelithiasis	Gallstones and gallbladder disease	Important genetic modifier is polymorphic (AT) repeats in promoter of UGT1AI gene.¹¹¹ Gall bladder disease in 28% at median age of 28y¹⁶
Retinopathy	Grade 1 —IV retinopathy	>30% of patients.¹¹² Prevalence higher in HbSC¹¹³